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Vol 70, No 4 (2019)
Review paper
Submitted: 2019-01-21
Accepted: 2019-02-03
Published online: 2019-08-14
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Current surgical management in RET mutation carriers [Aktualne postępowanie chirurgiczne u nosicieli mutacji proto-onkogenu RET]

Agnieszka Czarniecka1, Małgorzata Oczko-Wojciechowska2, Anna Hajduk2, Marcin Zeman1, Barbara Jarzab2
DOI: 10.5603/EP.a2019.0021
·
Pubmed: 31489961
·
Endokrynol Pol 2019;70(4):367-379.
Affiliations
  1. The Oncological and Reconstructive Surgery Clinic, M. Sklodowska-Curie National Research Institute of Oncology, Gliwice Branch, Poland
  2. Department of Nuclear Medicine and Endocrine Oncology, Maria Sklodowska-Curie Institute — Oncology Centre Gliwice Branch, Gliwice, Poland

open access

Vol 70, No 4 (2019)
Reviews — Postgraduate Education
Submitted: 2019-01-21
Accepted: 2019-02-03
Published online: 2019-08-14

Abstract

Medullary thyroid carcinoma (MTC) still remains a rare endocrine tumor. 20-25% of MTC cases are genetically determined. The detection of the RET proto-oncogene mutation in 1993 allowed to understand the unique genotype-phenotype relationships in hereditary medullary thyroid carcinoma (HMTC) and formed the basis for therapeutic decisions based on the molecular results. Currently, prophylactic thyroidectomy is a commonly adopted and accepted therapeutic method. The decision on the time and extent of surgery should be made based on the results of molecular examination, the assessment of calcitonin (Ct) concentration and family history. Treatment of patients with HMTC requires the cooperation of a multidisciplinary team of experts and should be done in specialized centers only. The study is a review of the current guidelines for surgical management in the MEN2 syndrome.

Abstract

Medullary thyroid carcinoma (MTC) still remains a rare endocrine tumor. 20-25% of MTC cases are genetically determined. The detection of the RET proto-oncogene mutation in 1993 allowed to understand the unique genotype-phenotype relationships in hereditary medullary thyroid carcinoma (HMTC) and formed the basis for therapeutic decisions based on the molecular results. Currently, prophylactic thyroidectomy is a commonly adopted and accepted therapeutic method. The decision on the time and extent of surgery should be made based on the results of molecular examination, the assessment of calcitonin (Ct) concentration and family history. Treatment of patients with HMTC requires the cooperation of a multidisciplinary team of experts and should be done in specialized centers only. The study is a review of the current guidelines for surgical management in the MEN2 syndrome.

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Keywords

RET mutations; MEN2 syndrome; surgery

About this article
Title

Current surgical management in RET mutation carriers [Aktualne postępowanie chirurgiczne u nosicieli mutacji proto-onkogenu RET]

Journal

Endokrynologia Polska

Issue

Vol 70, No 4 (2019)

Article type

Review paper

Pages

367-379

Published online

2019-08-14

Page views

2001

Article views/downloads

1668

DOI

10.5603/EP.a2019.0021

Pubmed

31489961

Bibliographic record

Endokrynol Pol 2019;70(4):367-379.

Keywords

RET mutations
MEN2 syndrome
surgery

Authors

Agnieszka Czarniecka
Małgorzata Oczko-Wojciechowska
Anna Hajduk
Marcin Zeman
Barbara Jarzab

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